Presentation
Previously fit and well, presented with rapidly progressive behavioral changes and upper limb myoclonus. CT showed no changes.
Patient Data
Brain MRI shows bilateral and symmetrical abnormal signal intensity in the basal ganglion, particularly evident within the head of the caudate nucleus and in the putamen.
The basal ganglia are hyperintense on T2, and more significantly on FLAIR. There is diffusion restriction involving these regions, characterized by hyperintensity on DWI, with corresponding decreased intensity on ADC maps.
There are bilateral and symmetrical focal hyperintensities on FLAIR/T2-weighted images, with no restricted diffusion, and elevated diffusivity on ADC, in the occipital cerebral cortex.
Conclusion:
The pattern of abnormality and diffusion restriction involving the basal ganglia is suspicious for Creutzfeldt-Jakob disease.
Case Discussion
Creutzfeldt-Jakob disease (CJD) forms a rare group of spongiform encephalopathies that results in rapidly progressive dementia and neurologic signs, with fatal outcomes 1-4. CJD is characterized by symmetrical/asymmetrical foci of signal abnormality and restricted diffusion in the caudate nuclei, putamina, and cerebral cortex 1-4. DWI, combined with FLAIR, is the best sequence to evaluate patients with CJD 1-4.
This case illustrates the typical clinical features, combined with positive CSF biomarker 14-3-3 protein, EEG and radiological findings of probable sCJD, with DWI giving the best clue to the diagnosis.
Case courtesy
- Sterfferson Morais, MD - PGY-3, radiology resident, Department of Radiology
- Tarcisio Brito Santos, MD - PGY-2, neurologist resident, Department of Neurology
- Marcelo Ataide de Lima, MD - neurologist, Department of Neurology
- Antonio Rodrigues de Aguiar Neto, MD - radiologist, Department of Radiology
Hospital da Restauração in Recife, Pe, Brazil
Unable to process the form. Check for errors and try again.